ISSN: 2165-3259

JAOCR

Official Journal of the American Osteopathic College of Radiology

CHEST IMAGING

Guest Editors: Les Folio, D.O., MPH, FAOCR Bernard F. Laya, D.O.

Editor-in Chief: William T. O’Brien, Sr., D.O.

April 2014, Vol. 3, Issue 2

JAOCR About the Journal

Aims and Scope The Journal of the American Osteopathic College of Radiology (JAOCR) is designed to provide practical up-to- date reviews of critical topics in radiology for practicing radiologists and radiology trainees. Each quarterly issue covers a particular radiology subspecialty and is composed of high quality review articles and case reports that highlight differential diagnoses and important teaching points.

Access to Articles All articles published in the JAOCR are open access online. Subscriptions to the journal are not required to view or download articles. Reprints are not available.

Copyrights Materials published in the JAOCR are protected by copyright. No part of this publication may be reproduced without written permission from the AOCR.

Guide for Authors Submissions for the JAOCR are by invitation only. If you were invited to submit an article and have questions regarding the content or format, please contact the appropriate Guest Editor for that particular issue. Although contributions are invited, they are subject to peer review and final acceptance.

Editor-in-Chief William T. O’Brien, Sr., D.O. San Antonio, TX

Design Editor Jessica Roberts Communications Director, AOCR

Managing Editor Tammam Beydoun, D.O. Farmington Hills, MI

Editorial Board Susann Schetter, D.O. Daniel J. Abbis, D.O. Les R. Folio, D.O. Michael W. Keleher, D.O. Rocky Saenz, D.O. Kipp A. Van Camp, D.O. John Wherthey, D.O.

J Am Osteopath Coll Radiol 2014; Vol. 3, Issue 2 Page i

Table of Contents

Chest Imaging

Guest Editors: Les Folio, D.O., MPH, FAOCR Bernard F. Laya, D.O.

Title/Author(s) Page No.

From the Editor 1

Review Articles Does This Belong to a Survivor of Childhood Cancer? Radiographic Findings Suggesting Previous Treatment for Childhood Cancer – A Review 2 Aswin V. Kumar, OMS3, Sue C. Kaste, D.O.

Interpretive Approach and Reporting the Intensive Care Bedside Chest X-Ray 12 Les Folio, D.O., MPH, FAOCR

Case Reports Cavitary Lung Mass in a Febrile Child 21 Rachel Pevsner Crum, D.O., Ricardo Restrepo, M.D., Nolan Altman, M.D.

Pulmonary Vascular Anomaly 25 David P. Concepcion, M.D., Bernard F. Laya, D.O., Ana Maria Saulog, M.D.

Interstitial Lung Disease 28 Shereef Takla, B.S., Aaron M. Betts, M.D.

Posterior Mediastinal Mass 32 Anagha Joshi, M.D., DMRE, Chintan Trivedi, M.D., DNB, Ashank Bansal, MBBS

JAOCR at the Viewbox Pulmonary Lymphangioleiomyomatosis 35 Bernard F. Laya, D.O., Regina C. Nava, M.D.

Hydatid Cyst of the Lung 36 Ali Yikilmaz, M.D.

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From the Guest Editor

In This Issue

Les Folio, D.O., MPH, FAOCR

Lead Radiologist for CT, Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD

When Lt Col (Dr.) O’Brien asked me congenital bronchopulmonary foregut to serve as guest editor for the first anomalies while highlighting a chest edition of JAOCR, I quickly particular case. involved our friend and AOCR Shereef Takla, BS, and Aaron M. colleague, Bernard Laya, DO. With his Betts, MD, tackled the challenge of expertise in chest imaging and as a interstitial lung disease, something world expert on tuberculosis in that I thought to be impossible in a children, combined with Bill O’Brien’s case report. Yet, they met and tireless dedication to make this exceeded their goal with great Journal a success, I felt as though we images, differentials, and discussion. were in great hands to develop a chest imaging edition worth keeping Ali Yikilmaz, MD, presents an interesting case that although on your bookshelf for reference. seemingly uncommon, could show up I am confident you will find the at our viewbox at any time. Knowing included topics informative, practical the water-lily sign associated with to your practice, and up-to-date. For hydatid cysts will help us make the example, “The Posterior Mediastinal diagnosis. Mass” case report by Anagha Joshi, Although I see cases of MD, DMRE, Chintan Trivedi, MD, DNB, lymphangioleiomyomatosis (LAM) and Ashank Bansal, MBBS, has a nearly every day, Bernard Laya, DO, representative biopsy proven mass with differentials and great and Regina C. Nava, MD, put LAM into "We choose to go the the needed perspective with discussion. moon in this decade representative lung and Similarly, Dr. Rachel Crum’s extrapulmonary findings. and do the other skillful description of a pediatric I sought Bill and Bernie’s advice cavitary lung mass is supported with things, not because on making my ICU chest x-ray article great images and differentials that useful to the majority of radiologists they are easy, but allow radiologists to approach this in this audience. I included the basics scenario more confidently. because they are of line and tube placement, hard..." Aswin V. Kumar, OMS3, and Sue pulmonary pathology, newer imaging C. Kaste, DO, provide a thrilling techniques, and tips on reporting. review of chest x-ray findings that Lastly, I would like to recognize -President John F. Kennedy should make one consider that the patient might be a childhood cancer Lt Col William T. O'Brien, Sr., USAF, survivor. Knowing these clues will MC, for pioneering and bringing the help radiologists identify the effects JAOCR to its current status. Having served with the Air Force myself for of both the cancer and its therapies. 20 years and the AOCR for nearly the Nathan David P. Concepcion, same amount of time on various MD, Bernard F. Laya, DO, and Ana committees, taking on the JAOCR is a Maria Saulog, MD, orchestrated an major undertaking and is the epitome astounding yet concise summary on of the quote I selected.

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Childhood Cancer, Kumar et al. Does This Chest Radiograph Belong to a Survivor of Childhood Cancer? Radiographic Findings Suggesting Previous Treatment for Childhood Cancer – A Review

a,b b-d Aswin V. Kumar, OMS3 , Sue C. Kaste, D.O.

aLincoln Memorial University, Harrogate, TN bDepartment of Radiological Sciences, Division of Diagnostic Imaging, Memphis TN cOncology, St. Jude Children’s Research Hospital, Memphis TN dDepartment of Radiology, University of Tennessee School of Health Sciences, Memphis, TN

Introduction The aim of this article is to provide an overview of selected radiographic manifestations of thoracic Advances in the detection, treatment, and findings that may be associated with previous supportive care of pediatric malignancies has allowed treatment for pediatric cancers and their late effects for improved long-term survival among childhood by providing an image-based approach to identifying cancer survivors. At present, the 5-year survival for unique radiographic characteristics that may be seen those diagnosed with a pediatric malignancy exceeds on chest radiographs obtained for reasons unrelated 1 80% with a 10-year survival rate of 75%. The to a history of previous childhood cancer. The risk increasing number of adult survivors of childhood factors for and prevalence of tumor recurrence and malignancies now approaches an estimated 360,000 secondary malignant neoplasms are well-described in individuals, allowing for more extensive studies of the the literature and will not be included in this pictorial delayed manifestations of adverse effects related to review. cancer treatment.1, 2 Medical conditions that persist or present in 5 or more years following treatment are referred to as late effects. Studies that investigate the Residual Mediastinal Mass After Treatment For late effects of pediatric cancer treatment have shown that 73.4% of survivors will experience a chronic Lymphoma medical condition, with over 40% experiencing a 3 The presence of residual abnormality of the serious or life-threatening problem. mediastinum or hila after completion of therapy for The manifestations of late effects are wide ranging lymphoma can induce anxiety in patients, parents, and and involve all organ systems, with differential healthcare providers. Approximately two-thirds of presentation largely dependent on both the primary patients with Hodgkin lymphoma and one-third of malignancy and the treatment received. Some of the patients with non-Hodgkin lymphoma have been most common late effects observed in childhood reported to have residual mediastinal masses after cancer survivors are pulmonary and cardiac completion of therapy,6 which can be apparent on complications, with skeletal complications and chest radiographs (Fig. 1). These residual masses more secondary malignancies being less common.4 The often occur in patients presenting with bulky increased survivorship and incidence of morbidity mediastinal disease7 or those with nodular sclerosing amongst those treated for childhood malignancies subtype of Hodgkin disease.8 The residual soft tissue necessitates increased vigilance on the part of the masses are usually composed of benign fibrotic or adult survivors’ health-care providers to both detect inflammatory tissue and may be seen in up to 41% of and treat the anticipatory late effects in this chest radiographs and 46% of chest CTs in pediatric population. The manifestations of tissue injury from patients treated for Hodgkin disease9; these masses therapy administered during childhood may not may calcify (Fig. 2).9 Typically, residual fibrotic masses become apparent until the patient enters a phase of continue to regress over time.7,8 rapid growth, such as adolescence. At such times, the Particularly in pediatric patients, thymic rebound, treatment insult on normal tissues may result in 5 developing after completion of therapy, may mimic a impaired growth. Diagnostic imaging can provide a residual mass.8 Comparison with prior chest imaging robust means through which many late effects can be can resolve whether or not the original mass has detected.

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A B C Figure 1. Residual Post-Therapy Mediastinal Mass. 18-year-old man diagnosed with Stage IV B nodular sclerosing Hodgkin disease was treated with chemotherapy and 2550cGy mantle and 800 cGy whole lung irradiation. Residual mediastinal mass persisted over the subsequent 8 years from initial imaging at diagnosis through follow-up. Posteroanterior chest radiograph at the time of diagnosis (A) shows a large anterior mediastinal mass which extends bilaterally from the midline. A follow-up posteroanterior chest radiograph obtained 8 years from diagnosis (B) shows residual superior mediastinal widening that corresponds to the residual masses shown on the corresponding computed tomography image (C).

Figure 2. Calcification of Residual Mediastinal Mass. 24-year-old survivor of Stage IIIB nodular sclerosing Hodgkin disease diagnosed at 15 years of age. His disease was refractory to standard chemotherapy, prompting autologous bone marrow transplantation and 2100 cGy mediastinal A B C radiation. Posteroanterior (A) and lateral (B) chest radiographs at the time of diagnosis show a bulky anterior mediastinal mass. Follow -up posteroanterior chest radiograph 6 years later (C) shows significant reduction in the mediastinal mass with development of dense calcifications. changed in size and contour. Increase in the residual chemotherapy-induced toxicities, all of which may mass or new adenopathy warrants further evaluation progress from initial injury to the pulmonary for the possibility of recurrent disease (Fig. 3). Such interstitium to over time.14 can be accomplished using MR10, 11 or CT for anatomic Pulmonary complications after therapy for childhood characterization of changes seen on chest cancer include pulmonary fibrosis (Fig. 4), chronic radiographs.6 However, MR and CT have limited ability , recurrent pneumonia, requirement for to differentiate between active disease and fibrosis or supplemental oxygen, and . Mertens, et al. scarring.9-13 Thus, 18F-FDG PET/PET-CT may be used to reporting on the prevalence of self-reported assess for metabolic activity (having largely replaced pulmonary complications from the Childhood Cancer 67Gallium imaging) that may indicate disease Survivor Study, found that chest radiation was relapse.6,13 statistically associated with all of these adverse late 14 effects, as were various chemotherapeutic agents. Chemotherapeutic agents associated with Pulmonary Complications development of pulmonary insufficiency include busulfan, carmustine14,15, cyclophosphamide, The lungs are one of the most radiation- and chemo- 15 14 lomustine, and bleomycin. At 20 years from sensitive organs in the body. Functional compromise diagnosis of the primary malignancy, a 3.5% arising from radiation is compounded by cumulative incidence of pulmonary fibrosis was

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Figure 3. Relapse Lymphoma. 9- year-old boy diagnosed with Stage IA Hodgkin disease right neck achieved complete remission with chemotherapy. At routine follow-up 3.5 years later, left hilar relapse was suspected. Posteroanterior (A) and lateral (B) chest radiographs show stable post-therapy A B C appearance of the thoracic structures. Follow-up posteroanterior chest radiograph (C) shows slight increased density left hilum which, on the lateral view (D), is shown to represent an ovoid nodule (lines). Axial non-contrast T1 (E) and T2 (F) weighted MR images of the chest show right paratracheal (long arrows), left hilar and subcarinal (short arrows) adenopathy, consistent with D E F disease relapse.

Figure 4. Progressive Radiation Fibrosis. 19-year-old woman diagnosed with Stage IIA nodular sclerosing Hodgkin disease was treated with chemotherapy and 2550 cGy modified mantle irradiation. One year after completing therapy, she developed disease relapse treated with intensive chemotherapy, autologous stem cell rescue, and radiation therapy to the lower cervical spine and porta A B C hepatis. At diagnosis, the posteroanterior chest radiograph (A) showed right paratracheal adenopathy and bilateral superior mediastinal widening, which improved with therapy. By 3 years later, posteroanterior chest radiograph (B) demonstrated straightening of the left mediastinum and early cephalad retraction of the left hilum. At 11 years, posteroanterior chest radiograph (C) showed progression of cephalad retraction of the left hilum and coarsening of post -radiation scarring. Imaging findings paralleled the patient’s decreasing pulmonary function, ultimately leading to her demise.

associated with chest radiation14, due to injury to type Cardiomyopathy II pneumocytes and endothelial cells.5,16 Chronic pulmonary impairment results from compromise of An increased risk for cardiovascular disease is seen alveolar growth and generation of new alveoli.5 in survivors of childhood cancer treated with radiation Radiographic findings of fibrosis include pleural therapy or chemotherapy, independently or in thickening, regional or focal pulmonary contraction, combination, and represents a cause of cardiac 21 linear scarring, and streaking that may extend beyond morbidity and mortality. Risk factors particularly the distribution of radiation portals.5 identified to increase the likelihood of developing anthracycline-associated cardiovascular toxicity The likelihood and severity of development of include age younger than 5 years at the time of pulmonary complications is dependent on the dose of 2 treatment, female sex, cumulative doses of 300 mg/m radiation and chemotherapy, younger patient age at or greater, cardiac irradiation of 3000 cGy or more, the time of therapy, and smoking.17 Pulmonary 18 and chemotherapy combined with radiation function longitudinally declines after therapy and 22,23 therapy. In addition, Orgel, et al. recently reported may compound the decrease in pulmonary function that an elevated body mass index and Hispanic normally seen with aging.19 Further, chemotherapy, ethnicity are also independent risk factors for the surgery, and bone marrow transplantation may development of declining left ventricular shortening compound the effects of radiation therapy.20 fraction in anthracycline-based therapy for acute

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Childhood Cancer, Kumar et al. myeloid leukemia.24 Other reported risk factors Breast Hypoplasia include black race and the presence of trisomy 21.25 Breast hypoplasia or aplasia is a well-known late The most common cardiac event reported is effect following irradiation to the chest during congestive .26 The hallmark of childhood (Fig. 6). Radiation-induced anthracycline cardiotoxicity is reduced thickness and underdevelopment of the breast has been reported in mass of the left ventricular wall.27 Though a variety of pathologies for which irradiation has been symptomatic cardiac compromise is infrequent22,23, a used, including cutaneous hemangiomas of the recent study reported a 12.6% incidence of such chest29, mediastinal lymphadenopathy30, Wilm events in patients treated with both anthracyclines tumor31, 32, and neuroblastoma.32 Radiation effects on and cardiac irradiation, 7.3% incidence with developing human breast tissue is dose dependent30, 33 anthracyclines alone, and 4.0% incidence after cardiac and may occur with doses of <500 cGy.34 Clinical irradiation with a median patient age of 27 years at changes associated with radiation-induced breast the time of the events26 (Fig. 5). Cardiotoxic effects of underdevelopment include the presence of therapy may not manifest until adulthood or during dyschromasia and telengiectasias on the affected times of stress, such as pregnancy or physical breast, as well as overall asymmetric breast exertion.22 development with the irradiated breast being smaller A recent investigation of 62 adolescent survivors of and irregular in size compared to the non-irradiated childhood cancer (mean age 14.6 years at the time of breast.33 Reported histopathological findings of study) who received anthracyclines as part of their irradiated hypoplastic breasts include extensive oncotherapy found that gadolinium-enhanced cardiac fibrosis, loss of breast lobules, and significant MR detected and quantified both left and right shrinkage of the ducts.33 Patients affected by breast 28 ventricular dysfunction in 61% and 27%, respectively. hypoplasia can also experience significant psychological distress due to the undesirable cosmetic effects of asynchronous breast growth.33 It is important to recognize the association of breast cancer arising as a result of irradiation that included

A B Figure 5. Anthracycline-Induced Cardiomyopathy. Figure 6. Breast Hypoplasia. 12-year-old patient diagnosed with nodular 49-year-old woman diagnosed with Wilm tumor at 5 years of age and sclerosing Hodgkin disease and received multiagent received 1200 cGy whole lung irradiation for pulmonary metastases, chemotherapy that included anthracycline. as well as 1200cGy abdominal radiation therapy for primary disease Pathologic examination revealed Grade 2 of 3 and hepatic metastases. Posteroanterior (A) and lateral (B) chest anthracycline cardiac toxicity. Posteroanterior chest radiographs demonstrate hypoplasia of both breasts. The radiograph obtained about 1 year after completion anteroposterior diameter of the chest is narrow from radiation- of therapy demonstrates cardiomegaly, bilateral induced rib dysplasia. pleural effusions, and pulmonary vascular congestion indicative of congestive heart failure.

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hypoplasia of bones exposed to radiation therapy, demineralization associated with chemotherapy and/ or radiation therapy, growth aberrations related to radiation therapy, and altered vertebral height when radiation therapy is compounded by the effects of chemotherapy.36 Similarly, chemotherapy can directly A affect growing bones.36,37 Growing bone is most susceptible to the effects of radiation during the two periods of most rapid growth: during the first 6 years of life and during puberty.38,39 Radiation injury is most likely related to injury of chondroblasts with inhibition of cartilaginous cells and is seen with single doses of 200 to 2000 cGy.5,40 Thus, the adverse impact of treatment – whether chemotherapy, radiation therapy, or in combination – on the developing skeletal structures varies with patient age, as well as B C the type, distribution, and intensity of therapy at the time of treatment.36, 39 Figure 7. Chest Wall Deformity And Scoliosis. At the age of 6 years, this boy was diagnosed with Ewing sarcoma family of tumors of the right chest wall. He received multiagent chemotherapy, surgical resection, and 504 cGy external beam Scoliosis irradiation. Over the course of 7 years, he developed significant scoliosis. Axial chest CT image at the time of diagnosis (A) shows Impaired vertebral growth can occur with doses of 41,42 38 the soft tissue mass with foci of increased attenuation arising 1000 to 2000 cGy and can lead to short stature , from the right lateral chest wall. Posteroanterior chest altered vertebral body configuration38,42, and radiograph obtained 2 years after completion of therapy (B) contribute to the development of scoliosis40,43,44 and/ shows chest wall deformity due to resection of several right 44 thoracic ribs and pulmonary scarring. Note the absence of a or kyphosis (Figs. 7 and 8). Probert and Parker visible scoliosis. Scoliosis series obtained 7 years after therapy reported changes in developing vertebral bodies when completion due to the presence of a “thoracic hump” (C) exposed to radiation doses of greater than 2000 demonstrates a 52 degree mid-thoracic convex right rads.38 Asymmetric exposure of the vertebral bodies rotoscoliosis. may contribute to the development of scoliosis.40,43,44 In addition to therapeutic irradiation, chest wall 35 breast tissue. After chest irradiation, the resection may result in scoliosis. In children, post- standardized incidence ratio for developing secondary surgical scoliosis is progressive and related to the breast cancer was 24.7 (95%CI 18.3-31.0), as opposed number of posterior ribs resected.45 to 4.8 (95%CI 2.9 - 7.4) for those who received no chest irradiation.35 Thus, education of these patients regarding health risks associated with chest irradiation Clavicular Growth should be prompted by recognition of this finding upon verification of prior therapy. Merchant, et al. investigated the effect of asymmetric exposure of the clavicles to 1500 cGy as administered with hemi-mini-mantle irradiation for Skeletal Sequelae unilateral Hodgkin disease of the neck or supraclavicular region. The clavicles which were fully Radiation-induced changes of bone have been exposed to radiation therapy grew 0.5 cm less overall recognized for decades, and any bone exposed to the compared to those only partially exposed (p=0.007), radiation field can be affected. Therapy inflicted during regardless of the patient’s age at the time of therapy the developmental stages of the skeleton can result in (median age, 13.3 years; range, 5.1 to 18.9 years)

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Figure 8. Rib Dysplasia. 49-year-old woman diagnosed with non-Hodgkin lymphoma at 9 years of age was treated with mediastinal radiation therapy and multiagent chemotherapy. Posteroanterior (A) and lateral (B) chest radiographs demonstrate linear pulmonary scarring with mild cephalad retraction of the hila (arrows) and a mild levoconvex mid- thoracic scoliosis (apex of the A B C curve indicated by the arrowhead). The striking chest wall deformity with depression of the anterior chest wall (B) resulted from radiation-induced rib dysplasia. Similarly, note the asymmetric size of the breasts (right smaller than left) and smaller volume of the right hemithorax compared to the left (A). The lateral view also readily demonstrates demineralization of the thoracic vertebral bodies. Initial posteroanterior ches t radiograph at the time of diagnosis C( ) shows extensive mediastinal, paratracheal, and right hilar adenopathy coupled with a large right pleural effusion.

B Figure 9. Asymmetric Clavicular Growth. 15-year-old boy was diagnosed with Stage IA Hodgkin disease of his right neck. In addition to multiagent chemotherapy, treatment included right hemi-mantle irradiation of 1500 cGy. Axial CT image of the neck at the time of diagnosis (A) shows prominent lymphadenopathy. Posteroanterior chest radiograph obtained 15 years after completion of treatment (B) A demonstrates asymmetric clavicular growth with the right clavicle measuring 2 cm shorter than the left.

(Fig. 9). Further, the effect on clavicular growth was following HSCT is 13.3 and 8.9 years, respectively.49, 50 more pronounced in the younger-aged patients (mean Among the risk factors investigated as contributing to age, 9.9 years) compared to those who were older their development following HSCT, only total body (mean age, 16.4 years; p=0.036).46 Thus, as with prior irradiation and a young age at time of TBI and or HSCT reports, the effects of radiation therapy on bone are have been consistently shown to significantly affect influenced by patient age, therapeutic dose, and the risk of developing osteochondromas.49,51 40 extent of tissues exposed. The prevalence of osteochondromas is approximately 3% in the general population with the majority presenting as solitary osteochondromas Radiation-Induced Exostosis unless in the setting of hereditary multiple exostosis.52 Among survivors of HSCT, approximately 1% develop Osteochondromas are the most common benign osteochondromas. Unlike the general population, only tumor of bone to occur following radiation therapy a slight majority of long-term survivors of HSCT (Fig. 10).47 They manifest as a late effect of total body develop solitary osteochondromas.49,51 In pediatric or local irradiation and have also been reported as a patients who undergo irradiation, damage to the long-term sequela of hematopoietic stem cell epiphyseal plate causes a portion of the epiphyseal transplantation (HSCT).48,49 The median age of cartilage to migrate to the metaphyseal regions presentation and latency for osteochondromas

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Childhood Cancer, Kumar et al. causing the formation of osteochondromas. other osseous lesions due to the contrast of high T2 52 Osteochondromas that occur as a result of and low T1 signal intensity of the cartilaginous cap. irradiation are radiographically indistinguishable from those that occur from other etiologies. Osteochondromas most commonly localize to the Demineralization metaphysis of long bones, particularly the femur and Survivors of childhood cancer are at risk for deficits proximal tibia, with involvement of flat bones being in bone mineral density which may lead to earlier less common.49 Clinically, osteochondromas present as onset and more severe osteoporosis and related painless slow-growing masses that cause local fractures.53 Attention to the integrity of bone distortion of tissue. Depending on their proximity to mineralization in the thoracic spine of childhood neurovascular structures, osteochondromas can cancer survivors is important. Occasionally, present with paresthesias or loss of peripheral pulse in compression fractures may be the first indication of the affected limb.52 In addition to the above such a deficit in survivors of childhood cancer. Though presentations, a minority of long-term survivors of the best studied pediatric cancer population has been HSCT are diagnosed with osteochondromas children treated for acute lymphoblastic leukemia, incidentally through the course of routine radiographic such deficits are associated with a variety of pediatric or clinical examination.49 malignancies, as well as with bone marrow Radiographically, the appearance of transplantation.54,55 osteochondromas can be described as cartilage Deficits in bone mineralization arise from a capped protruding osseous lesions that have cortical multitude of risk factors and include genetic and medullary contiguity with the parent bone. The predisposition54, lifestyle factors (such as suboptimal neck of an osteochondromas can either be wide or nutrition)53,54, inadequate weight-bearing exercise53,54, narrow, giving the appearance of either a sessile or treatment with osteotoxic chemotherapeutic agents pedunculated lesion, respectively.47 Osteochondromas (particularly glucocorticoids but also associated with can be easily recognized using radiographs. However, ifosfamide and methotrexate)37,53,54,56, more complex lesions, such as those that involve the endocrinopathies37,53,54, and radiation therapy whether spine or shoulder, can be better resolved with localized to the thoracic spine or gonads53, or cranial computed tomography.52 Magnetic resonance imaging irradiation.37,53 can accurately distinguish osteochondromas from

A B Figure 10. Radiation-Induced Exostosis. A 7-year-old girl returned 4 years after undergoing bone marrow transplantation for chronic myelogenous leukemia because of a newly found “lump” in her right anterior chest. The preparative regimen for her bone marrow transplantation included total body irradiation. Posteroanterior chest radiograph (A) was obtained, demonstrating expansion of the right anterior seventh rib (arrow). Axial limited chest CT (B) was performed through the rib for characterization of the abnormality shown on the chest radiograph and shows the typical appearance of an exostosis (skin marker).

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Figure 11. Osteonecrosis. 20-year-old woman diagnosed with B- cell non-Hodgkin lymphoma at 16 years of age experienced multiple relapses of the disease. She was treated with multiagent chemotherapy that included high dose glucocorticoids. The posteroanterior chest radiograph showed changes of osteonecrosis of the left humeral head. Dedicated radiographs of the shoulders confirmed the advanced osteonecrotic changes of both humeral headswit h crescent signs, collapse of the articular surfaces, and intermixed areas of sclerosis and cystic changes.

Osteonecrosis Acknowledgement

Children undergoing therapy for childhood cancer The authors would like to thank Ms. Sandra Gaither are at risk for osteonecrosis when treatment includes for manuscript preparation. high dose glucocorticoids, bone marrow transplantation, and/or local radiation (Fig. 11).57 The reported prevalence of osteonecrosis in these Disclosure survivors varies with the modality used to detect the Supported in part by grant P30 CA-21765 from the toxicity (MR being the most sensitive modality), National Institutes of Health, a Center of Excellence whether or not a report was based upon patients grant from the State of Tennessee, the Le Bonheur having symptoms, age at the time of diagnosis of the 58,59 Foundation (Memphis TN), and the American primary disease, and type of treatment. In contrast Lebanese Syrian Associated Charities (ALSAC). to the general population, osteonecrosis in survivors of childhood cancer occurs as a multijoint toxicity in 60% of those in whom it develops. As reported by the Childhood Cancer Survivor Study, the most frequent joints involved are the hips (72%), shoulders (24%) and knees (21%).58

Summary

The rapidly growing population of survivors of childhood cancer underscores the need for recognizing potential sequelae of both the primary disease and associated therapies, to include knowledge of risk factors for complications. While numerous reports are available regarding second malignant neoplasms in this population, only in the more recent past have investigations and understanding of adverse toxicities manifesting after completion of therapy been undertaken. It is with the hope of enhancing care of survivors of childhood cancer that this review of the more common chest manifestations has been developed. Though not meant to be all-inclusive, this work serves as a starting point to enhance the acumen of imaging healthcare providers, and thus, improve the care of these patients.

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Kremer LC, van Dalen EC, Offringa M, et al. Frequency and risk 4. Hudson MM, Ness KK, Gurney JG, et al. Clinical ascertainment factors of anthracycline-induced clinical heart failure in of health outcomes among adults treated for childhood children: a systematic review. Ann Oncol 2002:13:503-512. cancer. JAMA 2013:309:2371-2381. 24. Orgel E, Zung L, Ji L, et al. Early cardiac outcomes following 5. Friedman DL, Constine LS, Halperin EC, et al. Late Effects of contemporary treatment for childhood acute myeloid Cancer Treatment. In. Pediatric Radiation Oncology: Lippincott leukemia: a North American perspective. Pediatr Blood Williams & Wilkins; 2011. p 353-396. Cancer 2013:60:1528-1533. 6. Juweid ME. FDG-PET/CT in lymphoma. Methods Mol Biol 25. Krischer JP, Epstein S, Cuthbertson DD, et al. Clinical 2011:727:1-19. cardiotoxicity following anthracycline treatment for childhood 7. Radford JA, Cowan RA, Flanagan M, et al. The significance of cancer: the Pediatric Oncology Group experience. J Clin Oncol residual mediastinal abnormality on the chest radiograph 1997:15:1544-1552. following treatment for Hodgkin's disease. J Clin Oncol 26. van der Pal HJ, van Dalen EC, van DE, et al. High risk of 1988:6:940-946. symptomatic cardiac events in childhood cancer survivors. J 8. Luker GD, Siegel MJ. Mediastinal Hodgkin disease in children: Clin Oncol 2012:30:1429-1437. response to therapy. Radiology 1993:189:737-740. 27. Lipshultz SE. Exposure to anthracyclines during childhood 9. Brisse H, Pacquement H, Burdairon E, et al. Outcome of causes cardiac injury. Semin Oncol 2006:33:S8-14. residual mediastinal masses of thoracic lymphomas in 28. Ylanen K, Poutanen T, Savikurki-Heikkila P, et al. Cardiac children: impact on management and radiological follow-up magnetic resonance imaging in the evaluation of the late strategy. Pediatr Radiol 1998:28:444-450. effects of anthracyclines among long-term survivors of 10. Di CE, Cerone G, Enrici RM, et al. MRI characterization of childhood cancer. J Am Coll Cardiol 2013:61:1539-1547. residual mediastinal masses in Hodgkin's disease: long-term 29. Braun-Falco O, Schultze U, Meinhof W, et al. Contact follow-up. Magn Reson Imaging 2004:22:31-38. radiotherapy of cutaneous hemangiomas: therapeutic effects 11. Rahmouni A, Divine M, Lepage E, et al. Mediastinal and radiation sequelae in 818 patients. Arch Dermatol Res lymphoma: quantitative changes in gadolinium enhancement 1975:253:237-247. at MR imaging after treatment. Radiology 2001:219:621-628. 30. Kolar J, Bek V, Vrabec R. Hypoplasia of the growing breast 12. Nasr A, Stulberg J, Weitzman S, et al. Assessment of residual after contact-x-ray therapy for cutaneous angiomas. Arch posttreatment masses in Hodgkin's disease and the need for Dermatol 1967:96:427-430. biopsy in children. J Pediatr Surg 2006:41:972-974. 31. Macklis RM, Oltikar A, Sallan SE. Wilms' tumor patients with 13. Connors JM. Positron emission tomography in the pulmonary metastases. Int J Radiat Oncol Biol Phys management of Hodgkin lymphoma. Hematology Am Soc 1991:21:1187-1193. Hematol Educ Program 2011:2011:317-322. 32. Pinter AB, Hock A, Kajtar P, et al. Long-term follow-up of 14. Mertens AC, Yasui Y, Liu Y, et al. Pulmonary cancer in neonates and infants: a national survey of 142 complications in survivors of childhood and adolescent patients. Pediatr Surg Int 2003:19:233-239. cancer. A report from the Childhood Cancer Survivor Study. 33. Weidman AI, Zimany A, Kopf AW. Underdevelopment of the Cancer 2002:95:2431-2441. human breast after radiotherapy. Arch Dermatol 1966:93:708 15. Hudson MM, Mulrooney DA, Bowers DC, et al. High-risk -710. populations identified in Childhood Cancer Survivor Study 34. Furst CJ, Lundell M, Ahlback SO, et al. Breast hypoplasia investigations: implications for risk-based surveillance. J Clin following irradiation of the female breast in infancy and early Oncol 2009:27:2405-2414. childhood. Acta Oncol 1989:28:519-523. 16. Rubin P, Cassarett GW. Clinical Radiation Pathology. 35. Kenney LB, Yasui Y, Inskip PD, et al. Breast cancer after Philadelphia: W. B. Saunders Co; 1968. childhood cancer: a report from the Childhood Cancer 17. Liles A, Blatt J, Morris D, et al. Monitoring pulmonary Survivor Study. Ann Intern Med 2004:141:590-597. complications in long-term childhood cancer survivors: 36. Papadakis V, Tan C, Heller G, et al. Growth and final height guidelines for the primary care physician. Cleve Clin J Med after treatment for childhood Hodgkin disease. J Pediatr 2008:75:531-539. Hematol Oncol 1996:18:272-276. 18. Motosue MS, Zhu L, Srivastava K, et al. Pulmonary function 37. van Leeuwen BL, Kamps WA, Jansen HW, et al. The effect of after whole lung irradiation in pediatric patients with solid chemotherapy on the growing skeleton. Cancer Treat Rev malignancies. Cancer 2012:118:1450-1456. 2000:26:363-376. 19. Huang TT, Hudson MM, Stokes DC, et al. Pulmonary outcomes 38. Probert JC, Parker BR. The effects of radiation therapy on in survivors of childhood cancer: a systematic review. Chest bone growth. Radiology 1975:114:155-162. 2011:140:881-901.

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39. Dorr W, Kallfels S, Herrmann T. Late bone and soft tissue 51. Danner-Koptik K, Kletzel M, Dilley KJ. Exostoses as a long-term sequelae of childhood radiotherapy. Relevance of treatment sequela after pediatric hematopoietic progenitor cell age and radiation dose in 146 children treated between 1970 transplantation: potential causes and increase risk of and 1997. Strahlenther Onkol 2013:189:529-534. secondary malignancies from Ann & Robert H. Lurie Children's 40. Dawson WB. Growth impairment following radiotherapy in Hospital of Chicago. Biol Blood Marrow Transplant childhood. Clin Radiol 1968:19:241-256. 2013:19:1267-1270. 41. Mitchell MJ, Logan PM. Radiation-induced changes in bone. 52. Kitsoulis P, Galani V, Stefanaki K, et al. Osteochondromas: Radiographics 1998:18:1125-1136. review of the clinical, radiological and pathological features. 42. Neuhauser EB, Wittenborg MH, Berman CZ, et al. Irradiation In Vivo 2008:22:633-646. effects of roentgen therapy on the growing spine. Radiology 53. Kang MJ, Lim JS. Bone mineral density deficits in childhood 1952:59:637-650. cancer survivors: Pathophysiology, prevalence, screening, and 43. Parker RG, Berry HC. Late effects of therapeutic irradiation on management. Korean J Pediatr 2013:56:60-67. the skeleton and bone marrow. Cancer 1976:37:1162-1171. 54. Wasilewski-Masker K, Kaste SC, Hudson MM, et al. Bone 44. Makipernaa A, Heikkila JT, Merikanto J, et al. Spinal deformity mineral density deficits in survivors of childhood cancer: long- induced by radiotherapy for solid tumours in childhood: a term follow-up guidelines and review of the literature. long-term follow up study. Eur J Pediatr 1993:152:197-200. Pediatrics 2008:121:e705-e713. 45. DeRosa GP. Progressive scoliosis following chest wall 55. Kaste SC, Shidler TJ, Tong X, et al. Bone mineral density and resection in children. Spine (Phila Pa 1976 ) 1985:10:618-622. osteonecrosis in survivors of childhood allogeneic bone 46. Merchant TE, Nguyen L, Nguyen D, et al. Differential marrow transplantation. Bone Marrow Transplant attenuation of clavicle growth after asymmetric mantle 2004:33:435-441. radiotherapy. Int J Radiat Oncol Biol Phys 2004:59:556-561. 56. Wilson CL, Ness KK. Bone Mineral Density Deficits and 47. Murphey MD, Choi JJ, Kransdorf MJ, et al. Imaging of Fractures in Survivors of Childhood Cancer. Curr Osteoporos osteochondroma: variants and complications with radiologic- Rep 2013. pathologic correlation. Radiographics 2000:20:1407-1434. 57. Kadan-Lottick NS, Dinu I, Wasilewski-Masker K, et al. 48. Harper GD, cks-Mireaux C, Leiper AD. Total body irradiation- Osteonecrosis in adult survivors of childhood cancer: a report induced osteochondromata. J Pediatr Orthop 1998:18:356- from the childhood cancer survivor study. J Clin Oncol 358. 2008:26:3038-3045. 49. Faraci M, Bagnasco F, Corti P, et al. Osteochondroma after 58. Diller L, Chow EJ, Gurney JG, et al. Chronic disease in the hematopoietic stem cell transplantation in childhood. An Childhood Cancer Survivor Study cohort: a review of Italian study on behalf of the AIEOP-HSCT group. Biol Blood published findings. J Clin Oncol 2009:27:2339-2355. Marrow Transplant 2009:15:1271-1276. 59. Kaste SC, Karimova EJ, Neel MD. Osteonecrosis in children 50. Bordigoni P, Turello R, Clement L, et al. Osteochondroma after after therapy for malignancy. AJR Am J Roentgenol pediatric hematopoietic stem cell transplantation: report of 2011:196:1011-1018. eight cases. Bone Marrow Transplant 2002:29:611-614.

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Chest X-Ray, Folio Interpretive Approach and Reporting the Intensive Care Bedside Chest X-Ray

Les Folio, D.O., MPH, FAOCR

Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD

Introduction Approach to the Bedside Chest X-Ray

The chest x-ray (CXR) remains one of the most The recommended approach to CXR interpretation commonly requested imaging studies, yet is one of the is to first identify abnormal findings, including their most complex and least understood, particularly the location and distribution, and then further define intensive care unit (ICU) bedside examination. In patterns to help classify and categorize. This addition to deciphering numerous lines, tubes, lung represents the body of the report. Based upon this and pleural findings of the AP (anterior-posterior) information and correlation with any pertinent history, radiograph, critical care providers look to radiologists’ radiologists generate a differential diagnosis, or reports to summarize any pertinent changes in conclusion; that is, the impression section of the underlying pathological processes. This article will report. provide an overview of the bedside chest radiograph For example, the report body and impression of a in the ICU setting, as well as a guide to effective CXR describing a consolidation in a patient with cough, reporting for the radiologist. fever, and elevated white blood cell count may look While computed tomography (CT) has added like this: tremendous value in chest imaging in ICU patients, the Findings: A focal patchy opacity is noted in the CXR remains the mainstay in ICU imaging. Compared right upper lung field with air-bronchograms. to CT scans, the CXR can be obtained more readily and is associated with less radiation; thus, CXRs can be Impression: Consolidation on right, consistent with performed serially for temporal comparison. pneumonia Ultrasound is becoming more commonplace and can often be complementary to CXR, CT, and physiologic It is important to have a systematic approach to CXR 1 parameters. interpretation, especially when reviewing complex This article presents an overview of common CXR studies, such as the case shown in Fig. 1. One method findings in the ICU setting with example reporting, in uses a mnemonic-based search pattern consisting of the hope of increasing awareness of accepted report the ABCDEs twice. The acronym includes the following: terminology. It will also touch on traditional views, Airway (including an endotracheal [ET] tube, when new techniques/approaches to bedside chest imaging, applicable), Aorta (contours, edges, central lines), and technological advances that may improve (lungs and pleura), Bones (quick review diagnostic accuracy on CXR and negate the need for CT since this often does not significantly change in the in some conditions.2 ICU), Circulation (pulmonary vessels), Cardiac Topic points include positions of lines and tubes, (silhouette), Diaphragm (free air, costophrenic angles), abnormal collections of fluid and air, and common Deformity (post-operative, positioning considerations), causes of pulmonary opacities. Radiologists should Soft tissues (chest wall), Shoulders (periphery of keep in mind that ICU physicians want to know projection). Other search patterns include starting in findings that may alter management, those which are the midline and working one’s way outward or vice versa. The key point is to have a systematic search potentially life-threatening, as well as pertinent pattern which includes all aspects of the CXR and temporal changes. ensuring that the pattern is followed on each and every examination. This will help avoid becoming overwhelmed, especially when there are a multitude of findings.

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Figure 1. Example of a Potentially Perplexing Case Simplified Through a Systematic Approach and Routine Reporting. Starting with the ET tube, the tip is easily seen at the inferior medial clavicles within 5cm from the carina. Although the NG tube tip is not seen, the sideport is within the stomach; therefore, there is no need to say the tip is not seen. Next, describe the chest tubes and the Swan-Ganz catheter with its tip in right PA. Then move on to the tube effects, such as the subcutaneous emphysema, surgical clips, and extracardiac lucency. In evaluating the hemithoracies, the diaphragmatic silhouette is obscured, especially on the left due to a pleural effusion. Pulmonary opacities within the left hemithorax result from a pleural effusion with underlying parenchymal consolidation versus atelectasis. The cardiac silhouette and aortic contour are enlarged and show lucency on the left representing a pneumomediastinum. Finally, evaluate the extrathoracic regions of the film, including the upper abdomen, soft tissues, and bony structures.

Figure 2. Incorrect Positioning of an Endotracheal Tube. A CT scout image (A) shows an ET tube located within the left mainstem bronchus. Portable chest radiograph following repositioning of the ET tube (B) demonstrates correct positioning within the trachea with residual right lower lobe atelectasis from prior malpositioning; the region of atelectasis resolved on subsequent films (not shown). A B

Reports should be predictable and consistent for Support Devices: Lines and tubes critical care providers to quickly understand the meaning; for example, some radiologists report on The ICU generates many requests for support device lines and tubes first in the body, keeping that order in placement on CXR, especially serial examinations in the impression. This allows ICU staff and other patients with multiple lines and tubes. The American providers to know where to look for specific College of Radiology (ACR) appropriateness criteria information in reports. Standard reporting terminology provides guidelines for imaging in the setting of line 7 and formats for chest CT have been proposed, and the and tube placement. The literature supports Radiologic Society of North America (RSNA) has obtaining a CXR immediately following placement of existing and developing report templates in a variety endotracheal, enteric (especially feeding tubes), and of formats.3 The RadLex initiative, implemented by chest tubes; however, it does not support daily CXRs in RSNA, has led to standardized terms and reporting that the absence of a change in clinical condition or 8-10 may be useful in thoracic imaging.4,5 One group of suspected line or tube migration. researchers developed a structured report for the A comprehensive review of lines and tubes is 6 chest x-ray with standardized terminology. Although beyond the scope of this article. However, it is there is no national or international standard, there important to know which types of lines and tubes have seems to be a trend in this direction. been placed, along with their optimal locations and potential complications of malpositioning. Figs. 2, 3,

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Figure 3. Malpositioning of Central Venous Catheters. Portable chest radiograph flowing placement of a right jugular central line (A) shows an abnormal cephalad course of the catheter extending into the neck. Portable chest radiograph following placement of a left subclavian central line in a different patientB ( ) reveals the catheter coursing into a variant left superior vena cava.

A B

examinations when technologists have different degrees of inclination and thresholds for placing the “Upright,” “Erect,” or “Semierect” markers on the cassette. Rotation is often accentuated in bedside imaging compared to standard PA projections performed in the Radiology Department, making changes on serial exams more difficult to assess. Overlying material, such as the external component of tubes and lines, also limit visualization and evaluation of underlying structures. New technologies to improve CXR interpretation are available or in design and include dual energy, temporal subtraction, tomosynthesis, and decision support.11 Also available is line and tube visualization Figure 4. Positioning of Multiple Central Lines. software, which improves conspicuity of various Frontal chest radiograph reveals placement of bilateral support devices (Fig. 5).12,13 Some centers are using peripherally-inserted central venous catheters (PICC lines), as well as a right internal jugular (IJ) central venous catheter portable CT in the ICU, which has been found to be (CVC). The tip of the right IJ CVC is optimally located within advantageous due to minimizing the need for patient the distal superior vena cava. The left PICC line tip projects transport (especially with many support devices) and over the right atrium (arrows). obtaining more rapid assessments with superior spatial resolution.14 and 4 demonstrate examples of correct and incorrect Pleural Fluid, Air, and Loculations positioning of endotracheal tubes and central venous catheters. Chest tube positioning is discussed in the Pleural effusions are common in ICU patients. It is section covering pathology of the pleural space. important to distinguish effusions from other pathologic processes, as well as to assess for changes over time on serial examinations. Since fluid is Positioning, Technique Ideas and Technical dependent when not loculated or otherwise bound, Advances optimal positioning in as upright a position as possible is paramount. Inconsistent positioning over time often The portable CXR is often inconsistent and gives false impressions of changes in severity and has sometimes limited due to variable patient positioning variable effects on masking of underlying conditions, (rotation, tilt, angle of inclination, etc.). For example, it such as pneumonia or atelectasis. Technologists will can be difficult to evaluate pleural effusions on serial often indicate patient positioning with use of arrows or

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Figure 5. Application of the Line and Tube Visualization Software. In this patient who underwent right forequarter amputation from a scapular sarcoma, standard (A) and post-processed (B) CXR images were obtained. The post-processed image (B) more readily identifies the malpositioned left PICC line within the right brachiocephalic vein. There are also postoperative A B features to the lungs bilaterally with resultant volume loss to left lower lung field. Note a prototype inclination markern i the upper left portion of the image with demonstrates an inclination angle of 60 degrees. markers stating “Upright;” however, there is poor side of the effusion or what technologists often refer agreement or consistency as to when to use such to as “side down, side seen.” This means that a right- indications. For example, defining a threshold angle of sided effusion should be evaluated with a right side 60 degrees before an examination is to be considered down decubitus projection. This is opposite of the “Upright.” The angle of inclination also affects abdominal decubitus projection when looking for free- evaluation for pneumothoraces or free intra- air (“side up, side seen”) where a left lateral decubitus abdominal air. projection detects free air at the liver margin. The use of decubitus projections can be extremely Although this appears intuitive, it is important to be valuable in assessing the mobility and possibly the clear with the terminology when recommending, drainability of pleural fluid, often negating the need ordering, performing, or interpreting decubitus for CT (Fig. 6). One should keep in mind, however, that examinations. with the many support devices and critical nature of Empyemas represent localized infectious collections the patient’s underlying medical condition, decubitus within the pleural space and are not uncommon in the positioning in the ICU is often difficult and insome ICU setting. Differentiation from simple pleural cases not possible. The decubitus view to order is the effusions is a common and important question of ICU

A B C Figure 6. How the Decubitus Projection Can Determine Mobility of Pleural Effusions. Frontal (A) and lateral (B) chest radiographs demonstrate a moderate-sized right pleural effusion with suspected peripheral loculations. The right-side down decubitus view (C) verifies free mobility of the pleural fluid without evidence of loculations, thus negating the need for a CT examination.

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Figure 7. Empyemas. Frontal chest radiograph (A) reveals peripheral opacities along the lateral margins of the thoracic cavities, some of which have irregular borders. The size and extent of the collections were resulting in cardiac tamponade. Coronal reformatted CT image B ( ) better depicts the loculated collections which were subsequently drained but recurred.

A B

Figure 8. Lung Abscess. Frontal (A) and lateral (B) chest radiographs show a lung cavity with air-fluid level in the superior segment of the right lower lobe (right upper lung field) that has the same size and configuration on both the PA and lateral views, confirming a spherical shape. Biopsy revealed an aspergilloma in this patient with Job’s syndrome.

A B staff. However, the distinction cannot be made on CXR lower lung field often indicates the presence of a alone. Common findings include loculated, non-mobile hydropneumothorax. Treatment of pneumothoraces collections of pleural fluid (Fig. 7). A relatively specific often depends upon the size and pneumothorax and finding for empyemas includes air-fluid levels that are clinical status of the patient. Often times, placement of disparate in size/length when comparing the frontal a chest tube is necessary. 15 and lateral projections. This supports a non-spherical Chest tubes can be malpositioned in a manner shape and is helpful in distinguishing empyemas from 16 where bedside radiography alone is not adequate for simple effusions or pulmonary abscesses. evaluation.17 In this setting, CT is often necessary to Most parenchymal abscesses or cavities are evaluate for a malpositioned tube, typically when an spherical in shape, resulting in air-fluid levels of similar abnormal course is identified on CXR or the tube is not size/length on all projections (Fig. 8). Although there draining properly. Common findings associated with are findings suggestive of empyemas on CXR, malpositioning include visualization of the tube within recommending a CT and potentially image-guided a pulmonary fissure (Fig. 10) or in a superficial location drainage is often in order. with the sideports outside of the chest cavity. Visualization of air-fluid levels in the pleural space is Placement within the pulmonary parenchyma or the a useful finding in the setting of subtle pneumothoraces (Fig. 9). A dependent fluid level in the

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Figure 9. Air-fluid Level in the Pleural Space Indicative of a Hydropneumothorax. Frontal chest radiograph (A) demonstrates an air-fluid level within the inferior right hemithorax (thick arrows). Magnified view of the right upper thoracic cavity (B) reveals a subtle pneumothorax (thin arrows).

A

A B

Figure 10. Malpositioned Chest Tube. Frontal chest radiograph (A) shows bilateral chest tubes with persistent pneumothoraces. The chest tube on the right was not draining properly. Para coronal/axial (B) and sagittal reformatted C ( ) CT images demonstrate an interfissural course of the right-sided chest tube. A B C

Figure 11. Pneumonia. Frontal chest radiograph (A) demonstrates a focal rounded consolidation, likely within the lateral right middle lobe due to the lack of obscuration of the diaphragmatic border. Coronal reformatted (B) and axial (C) CT images confirm the middle lobe location and best depict the central air bronchograms in this patient with fever andX -linked agammaglobulinemia (XLA), which is the underlying cause of the lower lobe noted on the CT examination. chest wall is less common. atelectasis, especially on hypoinflated portable CXR, is a common dilemma for radiologists. When air space opacities are identified, differential conditions include Pulmonary/Lung Opacities pneumonia, , acute respiratory distress syndrome (ARDS), and pulmonary Pulmonary opacities are commonly seen in the ICU hemorrhage. setting. Distinguishing between air space disease and Atelectasis often mimics air space disease on CXR,

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Chest X-Ray, Folio since it often presents as a focal opacity with or pleural effusions. without air bronchograms. A key distinguishing feature Acute respiratory distress syndrome (ARDS) often is volume loss within the affected lobe, which may be occurs in the setting of shock or inhalation toxicity and subtle but is a useful discriminator. results in fluid accumulation within the lung The imaging pattern of infectious pneumonia is parenchyma. The air space disease may appear similar dependent upon the causative agent. Bacterial to that of pulmonary edema; however, it is not infections present with lobar air space disease, which typically associated with cardiomegaly or pleural may be localized or multi-focal (Fig. 11). effusions. The air space disease also tends to occur Parapneumonic effusions or empyemas may be seen. along the lung periphery.18 Chronically, ARDS may Atypical infections, such as viral or Mycoplasma, tend result in pulmonary fibrosis. to be interstitial but may occasionally be lobar as well. Pulmonary hemorrhage may be focal, especially in Nosocomial infections are more diffuse and aggressive the setting of trauma, or diffuse secondary toan with a higher prevalence in patients who are underlying systemic or autoimmune disease process. immunosuppressed. Cavitation is common and the Chest x-rays demonstrate multifocal air space morbidity and mortality is significantly higher than opacities, which may be ground glass, consolidated, community-acquired pneumonia. well-defined, or diffuseFig. ( 13). Cavitation is not Left-sided congestive heart failure demonstrates a uncommon.19 predictable sequence of findings on CXR (Fig. 12). Initially, there is enlargement of the cardiac silhouette with cephalization of pulmonary blood flow. As the Reporting/Terminology degree of heart failure progresses, interstitial edema is noted with prominent interstitial markings along the Findings in the body of the report should support periphery of the lung fields (Kerley B lines). Finally, the conclusions in the impression. A finding that edema extends into the pulmonary parenchyma and includes consolidation, for example, should have an pleural space, resulting in air space opacities and impression which includes a portion of the well-known

Figure 12. Congestive Heart Failure/Pulmonary Edema. Sequential frontal chest radiographs in a patient with progressive heart failure (A, B, and C) show an initial normal examination (A) with subsequent development of cardiomegaly, cephalization of pulmonary vasculature, and increased peripheral interstitial markings – referred to as Kerley B lines (B). Air space disease and pleural effusions are common and best depicted on images B and C. The A B relative increased lung volumes on image C are the result of interval intubation; enteric tubes and a right IJ CVC were also placed in this patient (C) and are appropriately positioned. A coronal reformatted image (D) nicely shows the peripheral Kerley lines, as well air space disease within the right lower lobe.

C D

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Figure 13. Diffuse Alveolar Hemorrhage. Frontal chest radiograph in a patient with known Kaposi Sarcoma involving the lungs (A) demonstrates multifocal interstitial and air space pulmonary opacities, as well as a left-sided pneumothorax. As the patient’s condition continued to deteriorate, subsequent imaging (B) shows extensive, diffuse pulmonary opacification. The support lines and tubes are appropriately positioned. Autopsy revealed diffuse alveolar hemorrhage.

Figure 14. Unsuspected Finding on CXR. A frontal radiograph of the chest and abdomen for line and tube placement (A) reveals an unusual bowel gas pattern compatible with pneumotosis intestinalis, which was confirmed on CT (B). The right-sided PICC line is in the SVC; the feeding tube tip is not seen, however, it courses well into the duodenum.

differential diagnosis of water, pus, blood, protein, and consistent and predictable. If the tip of the enteric cells based upon the clinical history. If there is a new tube is not seen but the sideport is visible, state the finding of a focal opacity and the critical care team is location of the sideport; there is no need to state that looking for infection, then pneumonia is most likely. If the tip is not seen in such cases. the opacity is diffuse, then fluid or blood (DAH) may be Although residents and fellows are often taught not added to the list of differentials. to use the term “infiltrate,” it may provide flexibility There are unique considerations in CXR terminology for both the radiologist and the ordering provider. For with regards to 2-dimensional representation of 3- example, if one commits to “consolidation” or “fluid,” dimensional structures. For example, there is this may minimize options for the clinician. Keeping considerable overlap and variability of lung lobes; the differential (impression) broader allows providers hence the use of the term lung “fields,” which is to apply the clinical information to their management. commonly used and appropriate. Also, if a central line However, there is a fine line between keeping tip appears low in the superior vena cava (SVC), differentials broad and being noncommittal or positioning and projection could mean that the tipis “hedging.” actually in right atrium; therefore, the broader phrase Having an organized search pattern is essential in “overlying the cavoatrial junction” may be useful. evaluating the entire film. It also helps prevent ICU staff, like radiologists, are busy and want “satisfaction of search” where obvious abnormalities concise information at their fingertips. The radiologist should avoid extraneous information and be

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Chest X-Ray, Folio are noted initially and more subtle findings are 1. Silva S, Biendel C, Ruiz J, et al. Usefulness of cardiothoracic overlooked.20 Fig. 14 shows an unsuspected case of chest ultrasound in the management of acute respiratory failure in critical care practice. Chest. 2013;144(3):859-65. pneumotosis intestinalis picked-up on a CXR for line 2. Rubinowitz AN, Siegel MD, Tocino I. Thoracic imaging in the and tube placement. ICU. Crit Care Clin 2007;23(3):539-73. Interaction With Intensive Care Team; The 3. Radiologic Society of North America (RSNA) radiology reporting initiative. Chest Radiography. http:// Report is Just the Beginning; ICU Rounds, Process www.radreport.org/specialty/ch, accessed Sep 2013. 4. RadLex, Radiology Society of North America (RSNA). Available Although the radiology report represents the final at http://www.radlex.com, accessed Aug 2013. product of a particular diagnostic imaging study, it may 5. Marwede D, Schulz T, Kahn T. Indexing thoracic CT reports using a preliminary version of a standardized radiological also represent the beginning of a diagnostic and lexicon (RadLex). J Digit Imaging 2008;21(4):363-70. procedural dialogue with ordering providers. A 6. Hasegawa Y, Matsumura Y, Mihara N, et al. Development of a continuous feedback and understanding of clinicians’ system that generates structured reports for chest x-ray needs provides insight that is otherwise not gained if radiography. Methods Inf Med 2010;49(4):360-70. radiologists remain in isolation. Breaking the 7. ACR Appropriateness Criteria; http://www.acr.org/~/media/ ACR/Documents/AppCriteria/Diagnostic/ misguided and stereotypical perceptions of RoutineChestRadiographsInICUPatients.pdf, accessed July radiologists keeping to themselves in a dark room will 2013. improve communication chains with ordering 8. Hejblum G, Chalumeau-Lemoine L, Ioos V, et al. Comparison providers and help guide the work-up and care for the of routine and on-demand prescription of chest radiographs in mechanically ventilated adults: a multicentre, cluster- most critically-ill patients. randomised, two-period crossover study. Lancet 2009; 374:1687-1693. 9. Graat ME, Choi G, Wolthuis EK, et al. The clinical value of daily Summary routine chest radiographs in a mixed medical-surgical intensive care unit is low. Crit Care 2006; 10(1):R11. The chest x-ray remains one of the most common, 10. Krivopal M, Shlobin OA, Schwartzstein RM. Utility of daily important, and complex examinations in the ICU routine portable chest radiographs in mechanically ventilated setting. Given the multitude of pathologies and patients in the medical ICU. Chest 2003; 123(5):1607-1614. 11. Jaeger S, Karargyris A, Candemir S, et al. Automatic screening support devices often encountered, it is critical that for tuberculosis in chest radiographs: a survey. Quant Imaging radiologists develop and follow a logical search pattern Med Surg 2013;3(2):89-99. to help define the underlying abnormalities, evaluate 12. Folio L. Chest Imaging; An algorithmic approach to learning. all aspects of the film, and avoid “satisfaction of New York: Springer, 2012, 136-37. search.” Correlating findings with the patient’s clinical 13. Foos DH, Yankelevitz DF, Wang X, et al. Improved visualization of tubes and lines in portable intensive care unit radiographs: status will aid in providing a useful list of differentials. a study comparing a new approach to the standard approach. Most importantly, continuous communication with Clin Imaging 2011; 35(5):346–352. ordering providers will allow radiologists to help guide 14. Teichgräber UK, Pinkernelle J, Jürgensen JS, et al. Portable computed tomography performed on the intensive care unit. Intensive Care Med 2003;29(3):491-5. The views expressed in this material are those 15. Bouros D (ed.). Pleural Disease, 2nd ed. New York: Informa, of the author, and do not reflect the official 2010, 35. policy or position of the U.S. Government or 16. Porcel JM, Light RW. Diagnostic approach to pleural effusion NIH. in adults. Am Fam Physician 2006;73(7):1211-1220. 17. Lim KE, Tai SC, Chan CY, et al. Diagnosis of malpositioned the work-up and management of the most critically-ill chest tubes after emergency tube thoracostomy: is computed tomography more accurate than chest radiograph? Clin patients. Imaging 2005;29(6):401-5. 18. Arsani A, Kaewlai R, Digumarthy S, et al. Urgent findings on portable chest radiography: what the radiologist should know – self-assessment module. Am J Roentgenol 2011; 196: WS37- 46. References 19. Primack SL, Miller RR, Müller NL. Diffuse pulmonary hemorrhage: clinical, pathologic, and imaging features. Am J Roentgenol 1995; 164: 295-300.

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Case Report, Crum Cavitary Lung Mass in a Febrile Child

Rachel Pevsner Crum, D.O., Ricardo Restrepo, M.D., Nolan Altman, M.D.

Department of Radiology, Miami Children’s Hospital, Miami, FL

Case Presentation

A 13-year-old boy with , gastroesophageal reflux (GERD), multiple food allergies, and history of 2 uncomplicated right middle lobe pneumonias within the last year presented to the emergency room with persistent cough and fever (max 102˚F) for 10 days, despite macrolide antibiotic treatment. A chest radiograph was performed in the emergency, followed by an esophagram/upper GI and CT examinations after admission (Fig).

A B

C D Figure. Chest radiograph on initial presentation (A) shows a large right upper lobe mass-like opacification with an air-fluid level. Also noted is a dilated esophagus (arrows). Esophagram performed 2 days later (B) shows diffuse, moderate dilatation of the esophagus and typical findings of achalasia with a “bird’s beak” appearance of distal esophagus (arrow) and persistent air-fluid level in the upright position. Despite IV antibiotic treatment, the patient did not improve clinically. Increasing size of the abscess on serial radiographs (not shown) was worrisome. Chest CT for further evaluation (C) shows enlargement of the right upper lobe consolidation with thick, irregular walls and an internal air-fluid level. The region of consolidation forms acute angles with the chest wall. Ultrasound-guided percutaneous drainage was performed; procedural chest radiograph following catheter placement and contrast injection (D) reveals confirmation of proper catheter placement. The infectious collection was successfully drained. Follow-up chest radiograph 8 weeks later showed resolution of the region of consolidation with a small residual linear opacity (not shown).

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Key Clinical Finding percutaneous drainage should be considered. Ultrasound guidance is preferred, as it allows for real- Recurrent pneumonia time localization of collections or abscesses. Direct visualization under ultrasound is advantageous for Key Imaging Findings catheter insertion and manipulation through septae or thick loculations that can interfere with drainage. In Cavitary mass with internal air-fluid level children, CT should not be used routinely; CT-guided drainage should be avoided, when possible, due to the

potential risks of ionizing radiation in the pediatric Differential Diagnoses population.2 Adjunctive tissue plasminogen activator (tPA) can be administered via a percutaneous catheter Lung abscess to promote drainage of an abscess by lysing fibrin Empyema strands.4 At our institution, we routinely use tPA for Necrotizing pneumonia drainage of loculated effusions and empyemas with good results, although use of tPA is controversial in the literature. One major complication of Discussion percutaneous drainage is the formation of a Although uncommon, complications from bronchopleural fistula; however, these may also occur pneumonia in children do occur, and recent literature directly from the complicated pneumonia alone. has suggested that they are increasing in prevalence.1 Tissue plasminogen activator is contraindicated if a Complications of pneumonia include parapneumonic bronchopleural fistula is present. Other major effusions, empyemas, lung abscesses, necrotizing complications of percutaneous drainage include pneumonia (multiple small abscesses), and empyema pneumothorax and hemorrhage. neccessitatis. These complications can lead to 1 extended hospital admission and increased morbidity. Pulmonary Abscess. Children usually recover completely without significant Pulmonary abscesses can be classified as primary or sequelae, unlike adults who often have underlying secondary. Primary pulmonary abscesses in a child are lung disease or co-morbidities.2 Although surgical usually a complication of pneumonia or aspiration.5 intervention is often required to treat adults with Secondary pulmonary abscesses can be caused by these same complications due to high associated underlying lung disease or pulmonary abnormality, mortality (20%), children often need only conservative either congenital or acquired. Secondary abscesses medical management.2,3 can also be seen in patients at risk of aspiration, such Ultrasound is invaluable for evaluation of the as those with neurodevelopmental abnormalities pediatric chest, as it involves no ionizing radiation, can (seizures, muscular dystrophy) or esophageal be performed bedside, and allows excellent evaluation abnormalities (achalasia, tracheoesophageal fistula, of simple or complex parapneumonic effusions and strictures).6 Underlying pulmonary disorders, such as abscesses. Ultrasound should be used as first-line cystic fibrosis or congenital lung malformation, are confirmation of a pleural effusion, as well as to guide also implicated as causes of recurrent pneumonia and treatment and need for percutaneous drainage, as it pulmonary abscesses.6 can readily distinguish between fluid, consolidations, Radiographs often show a large cavitary mass with and loculations.2 Ultrasound detects thin septae, thick walls; air-fluid levels may be seen. CT may not be fibrin strands, internal debris, and loculations in necessary on a routine basis, as radiographs may be complex effusions, which are usually not evident by sufficient for the diagnosis. Contrast-enhanced CT CT. CT should be reserved for complicated cases with may be useful for delineation and extent of disease worsening respiratory function or but should only be performed for worsening immunocompromised patients.2 respiratory symptoms. On CT, pulmonary abscesses For parapneumonic effusions and abscesses which are usually round with thick walls and irregular luminal are expanding or compromising respiratory function,

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Case Report, Crum surfaces. Vessels and bronchi terminate abruptly at visceral pleural. Ultrasound is important, as it can the abscess edge, and the walls of the abscess form distinguish fluid which is not readily defined on chest acute angles with the chest wall.7 On ultrasound, radiographs. Ultrasound of an empyema defines abscesses are seen as a thick-walled collections pleural thickening, loculations (which may have a containing echogenic pus and debris. Internal honeycombed appearance), fibrous strands, and septations and bright echogenic foci with dirty septae in the pleural space. shadowing from intraluminal air may be seen. Most empyemas can be treated conservatively with Lung abscesses in children often resolve with antibiotics and chest tube drainage.2 At our medical treatment alone. Usually, at least a 3-week institution, adjunctive tPA is used when draining course of intravenous antibiotics is needed with empyemas. Video-assisted thoracoscopic surgery is coverage for anaerobic organisms, which are most reserved for severe cases which do not respond to commonly implicated in pulmonary abscesses.8,9 conservative management. Within the current However, in some cases, the patient may require literature, there is still no clear consensus on surgical intervention with ultrasound-guided percutaneous treatment in children.1-3 drainage. Abscess drainage is indicated if the patient has persistent fever, sepsis, or worsening respiratory symptoms which are not responding to medical Necrotizing Pneumonia. treatment alone.2 Other indications include an Necrotizing pneumonia or cavitary necrosis is a enlarging abscess collection or imminent rupture into severe complication of pneumonia with destruction of a bronchus. Percutaneous ultrasound-guided drainage the lung parenchyma and gangrenous necrotizing of a recalcitrant pulmonary abscess is safe and changes; there may be formation of multiple small effective, avoids surgery, and helps to shorten the abscesses. Necrosis develops as a result of ischemia clinical course of the illness.5 caused by inflammation with occlusion of capillary vessels.6 Although the illness is severe, children

usually recover fully without the need for surgical Empyema. intervention or severe sequelae, contrary to what is Empyemas form as a complication of pneumonia typically seen in adults.6 and are characterized as complex collections of pus On chest radiographs, necrotizing pneumonia within the pleural space. Inflammation of the pleura appears as a large consolidation which may or may not leads to increased vascular permeability and fibrin contain small lucencies or cavities. CT is more production, resulting in pleural adhesions which can 3 sensitive than radiographs for evaluation of form a thick rind. Blockage of lymphatic drainage cavitation.6 Unlike pulmonary abscesses, necrotizing leads to increasing fluid accumulation, further pneumonia on CT demonstrates loss of the normal compressing and compromising the adjacent lung 3 lung architecture, decreased parenchymal parenchyma. Empyema formation evolves in 3 enhancement, and absence of a thick wall. On stages: 1) an exudative phase with inflammation and ultrasound, consolidated lung will have multiple small simple effusion of low cellular count; 2) a cystic and hypoechoic areas with decreased or only fibrinopurulent phase in which fibrin covers the pleura mild peripheral color flow. and forms thin septae and loculations; and 3) an organizing phase with formation of a thick fibrous capsule which prevents lung re-expansion.3 On radiographs, empyemas appear as loculated effusions with convex borders or consolidated lung. On CT, empyemas are often lentiform in shape, compress vessels and bronchi, and form obtuse margins with the chest wall.7 Uniform thickening of the visceral and parietal pleura form the “split pleural” sign previously described in the literature.7 Pleural enhancement is usually present and greatest along the

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Diagnosis References

Pulmonary abscess secondary to occult aspiration 1. Cohen E, Weinstein M, Fisman DN. Cost-effectiveness of from underlying esophageal achalasia. competing strategies for treatment of pediatric empyema. Pediatrics 2008; 121:e1250-57. 2. I M Balfour-Lynn, E Abrahamson, G Cohen, et al. BTS Summary guidelines for the management of pleural infection in children. Thorax 2005; 60: i1-21. In summary, children with recurrent pneumonia 3. Calder A, Owens CM. Imaging of parapneumonic pleural should receive follow-up imaging to document effusions and empyema in children. Pediatr Radiol 2009; resolution of the infectious process and exclude an 39:527-537. underlying pathologic process or mass. In our patient, 4. Grevais DA, Levis DA, Hahn PF, et al. Adjunctive Intrapleurral the underlying cause of recurrent pneumonia - and Tissue Plasminogen Activator Administered via Chest tubes Placed with Imaging Guidance: Effectiveness and Risk for ultimately abscess formation - was occult aspiration Hemorrhage. Radiology 2008; 246:956-963. due to primary achalasia. Although our patient had 5. Alsubie H, Fitzgerald DA. Lung Abscess in Children. Journal of been treated clinically in the past for gastroesophageal pediatric infectious diseases 2009; 4:27-35. reflux, an upper GI was not preformed until an 6. Donnelly LF, Klosterman LA. Cavitary Necrosis Complicating observant radiologist recommended the study for a Pneumonia in Children: Sequential Findings on Chest dilated esophagus noted on chest radiographs. Radiography. AJR 1998; 171:253-256. Pulmonary abscesses in children are commonly 7. Stark DD, Federle MP, Goodman PC, Podrasky AE, Webb WR. related to pneumonia or aspiration. Children usually Differentiating lung abscess and empyema: radiography and computed tomography. Am J Roentgenol 1983;141(1):163- have an excellent prognosis with conservative medical 167. management and no significant long-term sequelae. 8. Emanuel B, Shulman ST. Lung Abscess in infants & Children. Percutaneous ultrasound-guided drainage should be Clin Pediatr 1995 34:2-6. considered if clinical symptoms do not improve, the 9. Bartlett JG. Anaerobic bacterial infections of the lung and abscess enlarges, or there is impending rupture into a pleural space. Clin Infect Dis 1993; 16:S248–255. bronchus. First-line ultrasound should be considered to distinguish between complex and simple parapneumonic pleural effusions, as it is effective in delineating loculations and septations.

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Case Report, Concepcion et al. Pulmonary Vascular Anomaly

Nathan David P. Concepcion, M.D., Bernard F. Laya, D.O., Ana Maria Saulog, M.D.

Institute of Radiology, St. Luke’s Medical Center, Quezon City and Global City, Philippines

Case Presentation

A 32-year-old man presented with a two-year history of chronic cough and episodes of . He had been previously managed as having pulmonary tuberculosis. A chest CT was performed at another institution, which revealed a vascular malformation. CT angiography of the pulmonary arteries and aorta (Fig.) was advised and subsequently performed at our center.

A

LA

C

A B

Figure. Coronal reformatted images (A and B) show a large arterial branch emanating from the descending thoracic aorta (A) supplying the left lower lobe, as well as a prominent pulmonary vein normally draining into the left atrium (LA). Axial image in lung window (C) shows the abnormal vessels with hyperemia in the left lower lobe. No consolidation, soft tissue mass, or cyst is noted. 3-D reconstruction seen from the posterior view (D) shows the large systemic branch from the descending thoracic aorta (long arrow) and a hypoplastic pulmonary arterial supply (short arrow) to the left lower lobe.

D

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Key Clinical Finding as a plexiform mass of dilated vascular channels or with dilated, tortuous direct communication between Chronic cough with hemoptysis an anomalous feeding artery and draining vein (nidus). AVMs may be solitary or multiple. Multiple lesions are Key Imaging Findings often associated with hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber- 1,5 Pulmonary vascular anomaly Rendu syndrome. An AVM is excluded in our patient Systemic arterial supply to the left lower lobe with because of the presence of a systemic arterial supply prominent pulmonary venous drainage to the left lower lobe and the lack of a definite direct communication between the pulmonary artery and vein. Differential Diagnoses Pulmonary Varix. Pulmonary arteriovenous malformation Pulmonary varix refers to an enlargement of a Pulmonary varix segment of a pulmonary vein without an enlarged Pulmonary sequestration feeding artery or nidus. This is typically seen near the Pulmonary pseudosequestration left atrium with contiguity with the pulmonary vein. Varices may be congenital or acquired. Patients are

usually asymptomatic and generally not treated, but Discussion may also present with hemoptysis; hence, surgery may be required.5 Although the left inferior pulmonary vein Congenital bronchopulmonary foregut anomalies is prominent in our patient, the presence of a large may involve the lung parenchyma, airways, and/or systemic arterial supply makes this diagnosis unlikely. vascular arterial supply and venous drainage.1,2 These are included in a spectrum, which ranges from abnormal lung parenchyma with normal vasculature to Pulmonary Sequestration. abnormal vasculature with normal lung parenchyma. Pulmonary sequestration is characterized by In between are lesions with mixed parenchymal and 3,4,5 dysplastic, nonfunctioning lung parenchymathat does vascular abnormalities. not communicate with the tracheobronchial tree and Although chest radiographs play a role in the has an anomalous systemic arterial supply,1,2,3,4,5 incidental detection and initial imaging evaluation for usually from the thoracic or abdominal aorta; arterial such lung lesions, 5 CT is very useful in confirming the supply from the celiac, splenic, intercostal, subclavian, presence of a lesion, determining its extent, defining or even coronary arteries is less common.3,4 A associated abnormalities,1 and as pre-operative sequestration may appear as a persistent opacity or evaluation for surgical cases. 5 3-D and multiplanar mass. It may be associated with congenital pulmonary reformations can be particularly helpful in delineating airway malformation (CPAM), in which case air may be abnormalities of the bronchi and arterial and venous present within the lesion. Lesions may also contain air vasculature.1 when infected. The most common location is within 1,2,4 the left lower lobe. Sequestrations can be intralobar (within visceral pleura and venous drainage Pulmonary Arteriovenous Malformation. via the inferior pulmonary vein) or extralobar Pulmonary arteriovenous malformations (AVMs) (separate pleural covering with venous drainage usually present with dyspnea, hemoptysis, , or usually via systemic veins, typically the azygous vein clubbing; they may also be asymptomatic and found and less commonly via the portal, left subclavian, or incidentally. These lesions are caused by abnormal internal mammary veins).1,2,3,4,5 The imaging findings communication between the pulmonary arteries and in our patient are very similar to a pulmonary 1,2,5 veins and occur most frequently in the lower lobes. sequestration, except that the involved lung only CT may demonstrate more complex appearances, such shows hyperemia with a normal tracheobronchial tree.

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Pulmonary Pseudosequestration. Diagnosis Pseudosequestration is within the sequestration Pulmonary pseudosequestration spectrum and is characterized by lung parenchyma that is perfused by a systemic artery but maintains a normal tracheobronchial tree. The pulmonary artery is Summary often rudimentary or hypoplastic with poor arborization.6 Although the cause of the systemic With the advances in CT having multi-detector arterial supply is unknown, it is thought that technology, the evaluation and diagnosis of congenital persistence of an embryonic connection between the bronchopulmonary foregut malformations is greatly aorta and the pulmonary parenchyma leads to the enhanced. Thorough and careful assessment of the anomaly.7 airway, lung parenchyma, esophagus, arteries, and veins should be systematically analyzed in order to According to Yamanaka and colleagues,8 patients arrive at the correct diagnosis. The treatment plan is may range from 0 to 68 years of age and are dependent in the proper identification of the predominantly male. Pseudosequestration is often left pulmonary and systemic vessels. -sided and supplied by a branch of the descending thoracic aorta. The pulmonary veins drain normally In our 32-year-old patient who presented with a two into the left atrium. Most patients are asymptomatic; -year history of chronic cough and hemoptysis, CT when symptomatic, hemoptysis, exertional dyspnea, findings in the left lower lobe of a predominantly and congestive heart failure from left heart overload systemic arterial supply from the descending thoracic are the most common presentations.8 A cardiac aorta, a small pulmonary arterial supply, a prominent (continuous or systolic) murmur is the most common left inferior pulmonary vein draining into the left clinical manifestation in children.7,8 atrium, and a normal tracheobronchial tree leads to the diagnosis of pulmonary pseudosequestration. Based upon the above-mentioned characteristics, our patient has pulmonary pseudosequestration in the left lower lobe. References

1. Daltro P, Bradley LF, Donnelly LF, et al. CT of Congenital Lung Lesions in Pediatric Patients. AJR 2004; 183: 1497-1506. 2. Daltro P, Werner H, Gasparetto TD, et al. Congenital Chest Malformations: A Multimodality Approach with Emphasis on Fetal MR Imaging. RadioGraphics 2010; 30: 385-395. 3. Biyyam DR, Chapman T, Ferguson MR, et al. Congenital Lung Abnormalities: Embryologic Features, Prenatal Diagnosis, and Postnatal Radiologic-Pathologic Correlation. RadioGraphics 2010; 30: 1721-1738. 4. Newman B. Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol 2006; 36: 773–791. 5. Lee EY, Boiselle PM, Cleveland RH. Multidetector CT Evaluation of Congenital Lung Anomalies. Radiology 2008; 247: 632-648. 6. Singh AS, Subbain SK, Subramanian KG, et al. Pseudosequestration of the left lung. Tex Heart Inst J 2007; 34 (2): 195-198. 7. Do KH, Goo JM, Im JG, et al. Systemic Arterial Supply to the Lungs in Adults: Spiral CT Findings. RadioGraphics 2001; 21: 387-402. 8. Yamanaka A, Hirai T, Fujimoto T, et al. Anomalous systemic arterial supply to normal basal segments of the left lower lobe. Ann Thorac Surg 1999; 68: 332-338.

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Case Report, Takla et al. Interstitial Lung Disease

Shereef Takla, B.S.a, Aaron M. Betts, M.D.b

a Uniformed Services University of the Health Sciences, Bethesda, MD b Department of Radiology, University of Cincinnati Medical Center, Cincinnati, OH Case Presentation

A 76-year-old woman with a history of heart failure presented with 3 days of non-productive cough and increased dyspnea both at rest and with exertion. The patient had been admitted for community-acquired pneumonia 6 months earlier and has required supplemental home oxygen since that illness. She denied prior history of smoking or significant occupational/environmental pulmonary exposures. On clinical examination, she was afebrile with an oxygen saturation of 93% on room air. Pulmonary revealed mild respiratory at the lung bases. A high resolution CT of the chest was performed (Figs. 1-3) .

A B

C

Figure. Axial high-resolution computed tomography (CT) images through the chest (A and B) show septal thickening with a subpleural distribution and traction bronchiectasis. Fibrotic changes with honeycombing are noted at the posterior lung bases (B). Coronal reformatted CT image (C) demonstrates the basilar and subpleural distribution of the findings seen above. Mosaic ground glass attenuation is also noted .

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Key Clinical Findings high-resolution CT alone. However, correlation of the imaging appearance with patient’s medical history, Increased dyspnea with non-productive cough occupational/exposure history, and progression over Bibasilar inspiratory crackles time can often yield a final diagnosis.

Key Imaging Findings Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis. Interstitial lung disease with a peripheral basilar Idiopathic interstitial pneumonias are primarily distribution classified by histopathologic patterns and criteria. These patterns correlate with fairly characteristic findings on high resolution CT imaging of the chest. Differential Diagnoses Among the idiopathic interstitial pneumonias, usual Usual interstitial pneumonia interstitial pneumonia (UIP) is the most common histologic and imaging pattern. Idiopathic pulmonary Non-specific interstitial pneumonia fibrosis (IPF) is the prototypical and most common Interstitial lung disease associated with connective entity that corresponds to the morphologic pattern of tissue disease UIP. Asbestosis Patients with UIP/IPF are usually 50 years or older at the time of diagnosis. A history of smoking is associated with increased risk of IPF. Patients often do Discussion not respond to treatment with corticosteroids, resulting in a relatively poor prognosis. Patients with Interstitial lung disease (ILD) encompasses a broad UIP/IPF have a median survival ranging from 2 to 4 category of pulmonary diseases affecting the years after initial diagnosis.1,2 interstitium of the lung. Progressive dyspnea and cough are common presenting symptoms. The clinical On chest radiographs, UIP/IPF may appear normal assessment of patients with suspected ILD includes a or demonstrate decreased lung volumes with reticular thorough history, physical examination, and markings in an apicobasilar distribution with more pulmonary function testing. The radiologic evaluation advanced disease. High-resolution CT imaging shows includes chest radiographs and high-resolution CT. reticular opacities with a basilar and peripheral The information obtained from clinical and imaging (subpleural) predominance, honeycombing, and traction bronchiectasis. Ground glass opacities may evaluations may often yield a leading diagnosis 1,2 without the need for conformational surgical lung also be seen. biopsy. Progressive dyspnea, non-productive cough, and Non-Specific Interstitial Pneumonia. restrictive pattern on pulmonary function testing raise In the context of the morphologic pattern of UIP, clinical suspicion for an interstitial process. The non-specific interstitial pneumonia (NSIP) should be radiologic findings may confirm the presence of an considered. While the characteristic imaging findings interstitial process, and the specific findings and of NSIP are somewhat different from UIP, there is a distribution may further narrow the differential considerable overlap between the two conditions. diagnosis. In the context of high-resolution CT showing NSIP shows a similar pattern of subpleural reticular septal thickening with basilar and subpleural opacities with traction bronchiectasis. However, NSIP distribution, honeycombing, and traction tends to lack the apicobasilar gradient and bronchiectasis, the primary differential diagnosis honeycombing. Ground glass opacities are a more includes usual interstitial pneumonia, interstitial lung prominent feature of NSIP compared to UIP. Clinically, disease associated with connective tissue disease, and patients with NSIP tend to be slightly younger than asbestosis. A final diagnosis is often not possible by patients with UIP (age 40-50).

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The most important clinical distinction between UIP fibers are serpentine and amphiboles. As the name and NSIP is prognosis. The histologic pattern of implies, the serpentine fibers are curly and flexibile, cellular NSIP (predominantly inflammatory without while the amphiboles are straight, needle-shaped fibrosis) has a survival rate of nearly 100%, and the fibers. While both forms may lead to asbestos-related histologic pattern of fibrotic NSIP is associated with a 5 lung disease, the amphiboles are considered more -year survival rate of 45-90%. Compared to UIP, NSIP toxic. Exposure to asbestos fibers by inhalation can also shows a favorable response to treatment with lead to various manifestations of asbestos-related lung corticosteroids and cytotoxic agents.1-3 disease, including pleural effusion, pleural plaques (with or without calcification), diffuse pleural thickening, and/or malignant mesothelioma. Patients Connective Tissue Disease Associated Interstitial Lung with asbestos exposure are also at increased risk of Disease. primary bronchogenic carcinoma. Connective tissue diseases are a group of Asbestosis is a form of asbestos-related lung disease inflammatory autoimmune-mediated processes that that leads to interstitial fibrosis. Asbestosis is clinically may affect multiple organ systems, including the lung and histologically similar to idiopathic pulmonary parenchyma and chest cavity. Rheumatoid arthritis fibrosis. On high-resolution CT imaging, asbestosis will and progressive systemic sclerosis are two of the more show subpleural septal thickening and traction common connective tissue diseases that may result in bronchiectasis in a similar distribution as seen with interstitial lung disease with reticular opacities ina UIP/IPF; honeycombing is seen in more advanced basilar distribution. The interstitial lung disease that cases.6 Concomitant asbestos-related pleural disease develops with these entities may show a histologic may be seen in 75-83% of patient with asbestosis. pattern of UIP or NSIP, with UIP being more common However, in the absence of pleural abnormalities, in rheumatoid arthritis, and NSIP more common in asbestosis and idiopathic pulmonary fibrosis cannot be progressive systemic sclerosis. differentiated by imaging alone.7 The prevalence of interstitial lung disease in rheumatoid arthritis is variable, ranging from 5 to 40%. Interstitial lung disease is usually a late complication of the disease. Other common thoracic manifestations of rheumatoid arthritis include pleural thickening or pleural effusion. Rarely, cavitary necrobiotic rheumatoid nodules may be seen. Approximately 80% of patients with progressive systemic sclerosis will develop interstitial lung disease, and up to 97% of patients with progressive systemic sclerosis will have esophageal involvement of the disease, leading to esophageal dysmotility. On imaging, the esophageal involvement manifests as a patulous and fluid-filled esophagus. The coexistence of these common manifestations is highly suggestive of interstitial lung disease associated with progressive systemic sclerosis.4,5

Asbestosis. Asbestos is a non-combustible and durable silicate mineral that has been commercially developed for many purposes. The most common commercial applications include insulation material and brake pads/linings. The two main classifications of asbestos

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Case Report, Takla et al.

Diagnosis References

Usual interstitial pneumonia/idiopathic pulmonary 1. Mueller-Mang C, Grosse C, Schmid K, et al. What every fibrosis radiologist should know about idiopathic interstitial pneumonias. Radiographics 2007;27(3):595-615. 2. Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of Summary the ATS/ERS 2002 classification of idiopathic interstitial pneumonias. Radiographics 2003;23(5):1057-1071. High-resolution CT is an important component in 3. Kligerman SJ, Groshong S, Brown KK, et al. Nonspecific the evaluation of interstitial lung disease. When used interstitial pneumonia: radiologic, clinical, and pathologic in conjunction with a thorough history, physical considerations. Radiographics 2009;29(1):73-87. examination, and pulmonary function testing, high- 4. Capobianco J, Grimberg A, Thompson BM, et al. Thoracic resolution CT imaging may eliminate the need for manifestations of collagen vascular diseases. Radiographics 2012;32(1):33-50. surgical lung biopsy. Knowledge of subtle differences 5. Kim EA, Lee KS, Johkoh T, et al. Interstitial lung diseases in the imaging of various interstitial lung diseases may associated with collagen vascular diseases: radiologic and help narrow the differential diagnosis. However, there histopathologic findings. Radiographics 2002;22 Spec No:S151 is significant overlap in the imaging appearance of -165. various interstitial lung diseases, and a final diagnosis 6. Roach HD, Davies GJ, Attanoos R, et al. Asbestos: when the by imaging alone is not always possible. Rather, the dust settles an imaging review of asbestos-related disease. high-resolution CT findings must often be interpreted RadioGraphics 2002;22 Spec No:S167-184. in the context of patients medical, occupation, and 7. Akira M, Yamamoto S, Inoue Y, et al. High-resolution CT of exposure history. asbestosis and idiopathic pulmonary fibrosis. Am J Roentgenol 2003;181(1):163-169.

The views expressed in this material are those of the author, and do not reflect the official policy or position of the U.S. Government, the Department of Defense, or the Department of the Army.

J Am Osteopath Coll Radiol 2014; Vol. 3, Issue 2 Page 31

Case Report, Joshi et al. Posterior Mediastinal Mass

Anagha Joshi, M.D., DMRE, Chintan Trivedi, M.D., DNB, Ashank Bansal, MBBS

Lokmanya Tilak Municipal Medical College & Lokmanya Tilak Municipal General Hospital, Sion, Mumbai, India

Case Presentation

A 57-year-old woman presented with epigastric pain and cough for a period of 15 days, as well as a history of significant weight loss of 12 kgs in past 2 months. The patient had no significant past medical history. A chest x-ray was performed (Fig. 1), which prompted further work-up, consisting of a contrast-enhanced CT (Fig. 2) MRI (not shown), and PET-CT (Fig. 3). Frontal chest radiograph done as a part of the routine work up, revealed soft tissue opacity in the retrocardiac region, with the left heart border seen separate from the lesion. Lateral radiograph confirmed the posterior location of the mediastinal lesion.

Figure 1. Frontal radiograph (A) reveals a soft tissue opacity in the retrocardiac region with the left heart border seen separate from the lesion (black arrow). Lateral radiograph (B) confirms a posterior mediastinal location. The adjacent vertebral bodies and neural foramina appear normal.

A B

Figure 2. Post-contrast axial MIP CT image shows a heterogeneously enhancing mass in the posterior mediastinum engulfing the descending thoracic aorta, which shows multiple contrast-filled outpouchings. The fat plane with the liver is maintained, and the adjacent vertebral body does not appear to be involved. There is displacement of the esophagus and IVC.

Figure 3. Fused PET-CT image in the axial plane reveals intense uptake by the posterior mediastinal mass (SUV 7.3).

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Case Report, Joshi et al.

Key Imaging Finding characteristic. Necrosis, hemorrhage, and heterogeneous enhancement are more often seen Posterior mediastinal mass with malignant nerve sheath tumors. Sympathetic ganglion tumors, such as ganglioneuromas, are typically seen in the first decade of life, and are Differential Diagnoses vertically oriented along the lateral aspect of vertebrae, and are less common.2 Neurogenic Tumors / Peripheral Nerve Sheath Tumors Foregut Cysts Foregut Cysts. Lymphoma Mediastinal foregut cysts result from embryologic aberrations with anomalous budding of the primitive Aortic aneurysm foregut. The spectrum of anomalies includes Esophageal neoplasms bronchogenic cysts, esophageal duplication cysts, and Sarcoma neuroentric cysts. Bronchogenic cysts are most commonly located in the carinal region, while

esophageal duplication cysts are most often located Discussion along the esophagus in the lower mediastinum. Neuroentric cysts communicate with the meninges The posterior mediastinum is bounded anteriorly by and are usually associated with vertebral anomalies. the pericardium and great vessels, posteriorly by the Foregut cysts usually do not cause symptoms and have prevertebral fascia, and laterally by the pleura.1 Its similar imaging features. contents include the aorta, esophagus, azygous and Frontal radiographs typically reveal sharply hemiazgous vessels, neural structures, and lymph marginated areas of increased opacity. CT reveals well- nodes. By convention, the paravertebral space is also defined fluid density lesions, often with a thin included in the posterior mediastinum. Posterior enhancing wall. Occasionally, increased protein mediastinal masses can arise from any of these content will result in increased attenuation.1 There is structures. Morphological characteristics, no infiltration or invasion of adjacent structures and enhancement patterns, and relation to surrounding no solid tissue enhancement within the lesion. On organs as studied on imaging help in determining the MRI, foregut cysts appear as well-defined lesions organ of origin. Correlation with the clinical profile and which are hypointense on T1 and hyperintense on T2 histopathology are essential in arriving at the final weighted images. MR imaging is useful in determining diagnosis. These studies also play an important role in the cystic nature of foregut lesions with an atypical staging of the disease. appearance or increased attenuation on CT, since cysts will typically have T2 bright signal intensity, regardless Neurogenic Tumors. of the nature of the cyst contents. Neurogenic tumors, to include nerve sheath and sympathetic ganglion tumors, represent the most Lymphoma. common posterior mediastinal masses. Most lesions, Lymphoma presents as a soft tissue mass with especially if benign, are asymptomatic. Nerve sheath widening of mediastinum, which is often revealed on tumors, such as schwannoma and neurofibroma, are conventional radiographs. An anterior or middle most often seen in patients around 20-30 years of age. mediastinal location is more common than posterior. Frontal and lateral radiographs reveal widening of the CT demonstrates a lobulated soft tissue density mass neural foramina with splaying of ribs, which can be with heterogeneous enhancement; regions of confirmed on CT. These lesions demonstrate calcification may be seen post-treatment but are rare heterogeneous contrast enhancement on CT and in patients who have not undergone treatment. The appear hypointense on T1 and hyperintense on T2. margins often conform to surrounding structures. The MRI may show intraspinal extension, which is appearance of lymphoma on MRI varies based upon

J Am Osteopath Coll Radiol 2014; Vol. 3, Issue 2 Page 33

Case Report, Joshi et al. the type of lymphoma and whether or not the patient Diagnosis has undergone treatment.3 Sarcoma

Aortic Aneurysm. Patients with descending aortic aneurysms are Summary usually asymptomatic. Frontal radiographs reveal a Posterior mediastinal masses are usually posterior mediastinal mass with the aortic shadow not asymptomatic and are best diagnosed by cross seen separately from the lesion. Curvilinear sectional imaging. In this case, imaging findings were calcification may be seen at the periphery of the of a mass lesion with heterogeneous and delayed lesion. CT and MRA will show contrast enhancement in enhancement with erosion and aneurysm formation of the arterial phase. Partially thrombosed aneurysms the descending aorta, thereby favoring a neoplastic show regions of non-enhancing clot with areas of etiology. The tumor did not involve the esophagus or hemorrhage. vertebral bodies, and there was no intraspinal extension. Sarcoma, therefore, was the most likely Esophageal Neoplasms. diagnosis despite its rareity. A CT-guided biopsy revealed that this was a rare case of pleomorphic Patients with esophageal neoplasms generally undifferentiated sarcoma, formerly referred to as present with dysphagia to solid food and weight loss. malignant fibrous histiocytomas,4,5 with secondary Imaging findings often lag clinical presentation; involvement of the aorta. To our knowledge, only 13 therefore, radiographs may be normal initially. When cases of pleomorphic sarcoma involving the posterior large, esophageal tumors present as a posterior mediastinum have been described in literature.6 mediastinal soft tissue mass. CT shows eccentric or Hence, if the imaging findings don’t fit in any of the circumferential esophageal wall thickening with commonly known posterior mediastinal masses, a dilated proximal fluid and debris-filled esophageal differential of sarcoma should be kept in mind. lumen. Exophytic components may show heterogeneous enhancement. Invasion of local structures is common due to lack of a serosa.1 MRI provides little advantage over CT. References

Sarcoma. 1. Kawashima A, Fishman EK, Kuhlman JE, et al. CT of posterior Sarcomas are rare in the mediastinum and present mediastinal masses, RadioGraphics 1991;11:1045-1067 as posterior mediastinal masses, which may appear ill- 2. Nakazono T, White CS, Yamasaki F, et al. MRI findings of or well-defined on radiographs. Rib erosions may be mediastinal neurogenic tumors. Am J Roentgenol 2011;197:W643-52. seen. CT shows a solid mass lesion, often with infiltrative margins and heterogeneous enhancement. 3. Juanpere S, Cañete N, Ortuño P, et al. A diagnostic approach to the mediastinal massesA diagnostic approach to the MRI will show heterogeneous but predominantly T1 mediastinal masses Insights Imaging 2013;4(1):29-52. hypointense and T2 hyperintense signal intensity with 4. Fletcher CDM, Unni KK, Mertens F, eds. World Health foci of hemorrhage and necrosis. Neovascularity may Organization Classification of Tumors: Pathology and Genetics be present. It is uncommon for sarcomas to invade the of Tumours of Soft Tissue and Bone. Lyon, France, IARC Press, aorta and cause aneurysm formation. No specific 2002. imaging findings are known to differentiate the various 5. Fletcher CDM. Pleomorphic malignant fibrous histiocytoma: subtypes of sarcomas. Histopathology is essential for fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma. Am J Surg Pathol 1992;16 knowing the type of sarcoma. PET imaging helps in (3):213-28. grading and staging of the tumor. 6. Hernandez A, Gill, FI, Aventura E, et al. Mediastinal pleomorphic sarcoma in an immunodeficient patient: case report and review of the literature. Journal of the Louisiana State Medical Society 2012; 164(1): 21.

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JAOCR At the Viewbox JAOCR at the Viewbox

Bernard F. Laya, D.O.a,b, and Regina C. Nava, M.D.b

aInstitute of Radiology, St. Luke’s Medical Center, Quezon City, Philippines bInstitute of Radiology, St. Luke’s Medical Center Global City, Taguig, Philippines

B

A

C Pulmonary Lymphangioleiomyomatosis.

This chest radiograph of a 27-year-old woman presenting with severe difficulty of breathing (A) appears grossly unremarkable. A high resolution chest CT scan was obtained B( ), which reveals variably-sized cysts throughout the lung parenchyma with thickened interlobular septae, compatible with lymphangioleioyomatosis (LAM). Based upon these findings, an abdominal CT scan was performed C( ), revealing a large, heterogeneous right renal mass with predominantly fatty attenuation. A smaller lesion with similar imaging characteristics is seen in theleft kidney. Brain CT scan (not shown) demonstrated small subependymal calcifications. Overall constellation of findings is compatible with Tuberous Sclerosis. Pulmonary LAM is a rare disease affecting mostly women of child-bearing age. It also occurs in some patients with Tuberous Sclerosis. It is characterized by disorderly proliferation of smooth muscle throughout the lungs, causing destruction of lung tissue and leading to abnormal cyst formation. Dyspnea and pneumothorax are the two most common presenting symptoms. LAM is a slowly progressive condition with a poor prognosis. Treatment is difficult and is primarily supportive.

J Am Osteopath Coll Radiol 2014; Vol. 3, Issue 2 Page 35

JAOCR At the Viewbox JAOCR at the Viewbox

Ali Yikilmaz, M.D.

Istanbul Medeniyet University, Goztepe Research and Training Hospital, Department of Radiology, Istanbul, Turkey

B

A

Hydatid Cyst of the Lung.

A 12-year-old girl presented with cough and fever. PA chest X-ray shows a large air filled cyst in the left lung base (A). Contrast enhanced axial CT image (B) demonstrates a round cyst with air-fluid level and detached- floating germinative membranes (asterisk), which are typical for hydatid disease. Echinococcosis or hydatid disease is a parasitosis caused by infestation with Echinococcus granulosus (dog tapeworm). Although most children with pulmonary involvement by hydatid disease are asymptomatic, they may occasionally present with fever, , cough, and/or , which is usually a sign of cyst rupture. Diagnosis of hydatid disease depends on the combination of imaging findings and serology tests that use antigens specific for the organism. The radiological findings are characterized by single or multiple (~25%), round or oval-shaped, cystic nodules or masses (1-20 cm in diameter) with well-defined walls, surrounded by normal lung parenchyma. Other findings include an air- when a cyst communicates with a bronchus or the “water-lily sign” when a cyst membrane floats in residual fluid after the rupture of cyst. The water-lily sign is considered to be highly specific or pathognomonic, especially in endemic areas.

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